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pulm cram deck > SM 166a - Congenital and Pediatric Disorders > Flashcards

SM 166a - Congenital and Pediatric Disorders Flashcards

1
Q

You suspect an airway abnormality in your 3 month old patient. You can hear stridor when they breathe, and bronchoscopy reveals an omega-shaped epiglottis.

What is the most likely diagnosis?

How is it treated?

A

Laryngomalacia

Evaluate for aspiration and reflux, treat if necessary

The laryngomalacia may resolve on its own, surgery may be necessary if severe

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2
Q

What defines bronchopulmonary dysplasia?

How is severity defined?

A

Continued airway and parenchymal abnormalities in an infant with RDS.

Severity is defined by the amount of supplemental oxygen required at:

36 week post-menstrual age (if the baby was born at <32 weeks gestational age)

OR

56 DOL if (if the baby was born ≥32 weeks gestational age)

  • Mild
    • Breathing room air
  • Moderate
    • Breathing <30% FiO2
  • Severe
    • Breathing >30% FiO2 or requiring positive pressure ventilation
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3
Q

At what stage of embryonic development do alveolar cells differentiate into type 1 and type 2?

A

Saccular stage (24-36 weeks/birth)

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4
Q

What causes laryngomalacia?

A

Floppiness/softening of the larynx

Any of the following are possible:

  • Omega-shaped epiglottis
  • Short aryepiglottic folds
  • Prolapsed arytenoids
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5
Q

What abnormality is shown in this image?

A

Vascular sling: aberrant left pulmonary artery

The arrow is pointing to the left artery that is sneaking between the esophagus and the trachea

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6
Q

How is a vascular ring or sling diagnosed?

A

Barium swallow

Will show compression of the esophagus or bronchial tree

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7
Q

What congenital lung malformation is shown in this x-ray of a 2-month old girl?

A

Congenital pumonary aiway malformation (CPAM)

The arrow is pointing to a large systic mass

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8
Q

What congenital lung malformation is shown in these pictures?

A

Tracheo-Bronchomalacia

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9
Q

What are the risk factors for bronchopulmonary dysplasia?

A
  • Low birth weight
  • Low gestational age
  • Infections (maternal and infant)
  • Positive pressure ventilation
  • O2 toxicity
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10
Q

What is the treatment for laryngomalacia?

A

Evaluate for aspiration and reflux, treat if necessary

The laryngomalacia may resolve on its own, surgery may be necessary if severe

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11
Q

What is a bronchopulmonary foregut malformation?

A

Abnormal lung tissue is connected to the GI tract

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12
Q

What are ongenital pulmonary airway malformations?

A

A heterogeneous group of congenital cystic and non-cystic lung masses characterized by extensivve overgrowth of the primary bronchioles in communication with the abnormal (lacks cartilage) bronchial tree

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13
Q

Why are mothers at risk for preterm delivery prescribed steroids prior to 34 weeks?

A

To decrease the infant’s risk of bronchopulmonary dysplasia

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14
Q

What is congenital lobular emphysema?

A

Overinflation or distension of one or more pulmonary lobes

May have intrinsic or extrinsic causes

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15
Q

What congenital lung malformation is shown in this CT scan of a 2-month old girl?

A

Congenital pumonary aiway malformation (CPAM)

The arrow is pointing to a large systic mass

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16
Q

What is an intralobular pulmonary sequestration?

A

Extra lung tissue si sompletely covered by normal lung tissue OR by a segment of visceral pleura of the associated lung lobe

May be congenital or acquired

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17
Q

What is respiratory distress syndrome (RDS) in newborns?

A

A newborn has RDS if their lungs do not have enough surfactant

  • Often seen in premature infants
  • Surface tension increases
  • Compliance decreases
  • Breathing requires more effort
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18
Q

Describe the clinical presentation of a pumonary AVM

A

Asymptomatic hypoxia

OR

Bleeding in the lungs (pumonary hemorrhage, hemoptysis

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19
Q

List 3 congenital airway abnormalities

A

Laryngomalacia

Tracheo-bronchomalacia

Tracheoesophagel fistula (TEF)

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20
Q

How is a pumonary AVM diagnosed?

A

chest x-ray or angiography

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21
Q

How is a pulmonary AVM treated?

A

Embolization if it is large enough; the vessel is occluded so blood does not enter the AVM

22
Q

What is the treatment for tracheo-bronchomalacia?

A

Surgery if severe

23
Q

List the 3 major types of pulmonary sequestration

A
  • Intralobular pulmonary sequestration (most common)
  • Extralobular pulmonary sequestration
  • Bronchopulmonary foregut malformation
24
Q

What are the etiologies of intralobular pulmonary sequestration?

A
  • Congenital
    • ​Sequestration arises from the lung bud during development
  • Acquired
    • ​Sequestration develops after bronchial obstruction
      • ex: pneumonia -> no perfusion if no ventilation -> parasitation of pulmonary arteries
25
Q

Describe the presentation of tracheo-bronchomalacia

A
  • Noisy breathing caused by trachea and mainstem bronchi floppiness
    • Lack of C-shape to the airways
26
Q

What congenital lung abnormality is present in this CT scan of a 2-day old girl?

A

Congenital lobar emphysema

Note: hyperinflated LUL with attenuated lung markings, mediastinal shift to the right side

27
Q

What abnormality is shown in this image?

A

Double aortic arch - a type of vascular ring around the esophagus and bronchial tree

28
Q

What is a pulmonary atriovenous malformation (AVM)?

A

A pulmonary AVM is an abnormal connection between arteries and veins that causes blood to skip the capillary bed

(Basically a shunt)

29
Q

What congenital lung abnormality is present in this x-ray of a 2-day old girl?

A

Congenital lobar emphysema

Note: hyperlucency in the LUL

30
Q

Where are extralobular pulmonary sequestrations most commonly located?

A

Left lower lobe

Some (~10%) are below the diaphragm

31
Q

What is pulmonary seqestration? (congenital lung abnormality)

A

Normal, non-functioning lung tissue w/o a connection to the bronchial tree

Recieves blood from the systemic circulation

32
Q

What factors decrease an infant’s risk of bronchopulmonary dysplasia?

A
  • Antenatal steroids given to the mother prior to delivery
  • Surfactant therapy for the infant
  • Nutritional support to increase weight
  • Ventilator management
    • Don’t overventilate! Positive pressure ventilation and O2 toxicity increase an infant’s risk of BPD
33
Q

What is the difference between a vascular ring and a vascular sling?

A
  • Ring
    • Completely surrounds the esophagus and bronchial tree
    • Squishes teh trachea and esophgaus together
  • Sling
    • Wraps around the esophagus and bronchial tree
    • Must sneak back between the trachea and the esophagus
34
Q

What congenital lung malformation is pictured?

A

Tracheoesophagel fistula (TEF)

35
Q

What lung abnormalityis indicated by the arrow?

A

Intralobular pulmonary sequestration

36
Q

Describe the clinical presentation of a vascula ring or sling

A
  • Trouble swallowing
  • Stridor
  • Respiratory distress
  • Recurrent pneumonea
  • Apnea
37
Q

Describe the circulation of an intralobular pulmonary sequestration

Blood Supply:

Blood Drainage:

A

Blood supply: Systemic circulation (ascending or abdominal aorta)

Blood Drainage: Pulmonary veins

38
Q

How are vascular rings and slings treated?

A

Surgery

39
Q

Describe the circulation of an extalobular pulmonary sequestration

Blood Supply:

Blood Drainage:

A

Blood supply: Systemic circulation (ascending or abdominal aorta)

Blood Drainage: Systemic venous circulation (azygous or portal)

40
Q

What causes tracheo-bronchomalacia?

A

Floppiness in the trachea and mainstem bronchi

Lack of C-shape to the airways

41
Q

How is tracheoesophageal fistula diagnosed?

A
  • A tube cannot pass from the mouth to the stomach
  • Radiography: no shadow from the esophagus
42
Q

What is the etiology of extralobular pulmonary sequestration?

A

Usually congenital

43
Q

What congenital lung malformation is shown in this CT scan of a 1-day old boy?

A

Congenital pumonary aiway malformation (CPAM)

44
Q

What is a vascular ring or sling?

A

Abberations in the mediastinal vasculature caused by alterations in the aortic arch system that cause compression of the bronchial tree.

45
Q

Which lobe is most frequently affected by congenital lobar emphysema?

A

Upper left lobe

Congenital lobar emphysema is rarely bilateral or multifocal

46
Q

Describe the presentaton of tracheoesophageal fistula (TEF)

A

At DOL 1:

  • Infant can’t handle secretions: they drool a lot
  • Choking with feeding
47
Q

Describe the presentation of laryngomalacia

A
  • Stridor caused by an omega-shaped epiglottis
  • Short aryepiglottic folds
  • Prolapsed arytenoids

Overall floppiness/softness of the larynx

48
Q

What is an extralobular pulmonary sequestration?

A

An accessory lung covered by visceral leura, separated from the functional lung

49
Q

What is the treatment for tracheoesophageal fistula?

A

Surgery to disconnect the trachea and esophagus

50
Q

What causes congenital lobar emphysema?

A
  • Intrinsic
    • Weakness/absence of underlying bronchial cartilage causes the lobe to collapse
  • Extrinsic
    • Mass effect: pushing on the bronchi means that air cannot get out

Either etiology can lead to bronchial narrowing, air trapping, and overinflation/distension fo the lung

51
Q

Name 2 congential lung malformations that are vascular abnormalites

A
  • Vascular rings/slings
  • Pulmonary atriovenous malformations

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